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Craniopharyngioma

Definition

A craniopharyngioma is a noncancerous (benign) tumor that develops at the base of the brain near the pituitary gland (a small endocrine gland).

Causes

This tumor most commonly affects children 5 to 10 years of age. Adults can sometimes be affected. Boys and girls are equally likely to develop this condition.

Symptoms

Craniopharyngioma causes symptoms by:

  • Increasing pressure on the brain, usually from hydrocephalus
  • Disrupting hormone production by the pituitary gland
  • Decreasing vision due to pressure or damage to the optic nerve

Increased pressure on the brain causes headache, nausea, vomiting (especially in the morning), and difficulty with balance.

Damage to the pituitary gland causes hormone imbalances that can lead to excessive thirst, excessive urination, and stunted growth.

When the optic nerve is damaged by the tumor, vision problems develop. These defects are often permanent, and may get worse after surgery to remove the tumor.

Behavioral and learning problems may be present.

Most patients have at least some vision problems and evidence of decreased hormone production when they are diagnosed.

Exams and Tests

The doctor or nurse will examine you. Tests that may be done include:

  • Blood tests to measure hormone levels
  • CT scan or MRI scan of the brain
  • Neurological examination

Treatment

Traditionally, surgery has been the main treatment for craniopharyngioma. However, radiation treatment instead of surgery or along with a smaller surgery may be the best choice for some patients.

In tumors that cannot be removed completely with surgery alone, radiation therapy is usually necessary. If the tumor has a classic appearance on CT scan, a biopsy may not be necessary if treatment with radiation alone is planned.

Stereotactic radiosurgery is performed at some medical centers.

This tumor is best treated at a center with experience in treating patients with craniopharyngiomas.

Outlook (Prognosis)

In general, the outlook for patients with craniopharyngioma is good. Patients have an 80 to 90% chance of a permanent cure if the tumor can be completely removed with surgery or treated with high doses of radiation. If the tumor returns, it will usually come back within the first 2 years after surgery.

A patient's outlook depends on several factors, including:

  • Whether the tumor can be completely removed
  • What nervous system problems and hormonal imbalances the tumor and treatment cause

Most of the problems with hormones and vision do not improve with treatment. Sometimes the treatment may even make them worse.

Possible Complications

A large percentage of patients have long-term hormone, vision, and nervous system problems after craniopharyngioma is treated.

When the tumor is not completely removed, the condition may return.

When to Contact a Medical Professional

Call your health care provider for the following symptoms:

  • Headache, nausea, vomiting, or balance problems (signs of increased pressure on the brain)
  • Increased thirst and urination
  • Poor growth in a child
  • Vision changes

References

Maity A, Pruitt AA, Judy KD, Phillips PC, Lustig R. Cancer of the central nervous system. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Abeloff's Clinical Oncology. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2008:chap 70.

Review Date:7/30/2014
Reviewed By:Luc Jasmin, MD, PhD, Department of Surgery, Providence Hospital, Medford, OR, Department of Neurosurgery a Cedars-Sinai Medical Center, Los Angeles, CA, and Department of Oral and Maxillofacial Surgery at UCSF, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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Outcome Data

No data available for this condition/procedure.

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